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Orphanet Journal of Rare Diseases Aug 2011VACTERL/VATER association is typically defined by the presence of at least three of the following congenital malformations: vertebral defects, anal atresia, cardiac... (Review)
Review
VACTERL/VATER association is typically defined by the presence of at least three of the following congenital malformations: vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities. In addition to these core component features, patients may also have other congenital anomalies. Although diagnostic criteria vary, the incidence is estimated at approximately 1 in 10,000 to 1 in 40,000 live-born infants. The condition is ascertained clinically by the presence of the above-mentioned malformations; importantly, there should be no clinical or laboratory-based evidence for the presence of one of the many similar conditions, as the differential diagnosis is relatively large. This differential diagnosis includes (but is not limited to) Baller-Gerold syndrome, CHARGE syndrome, Currarino syndrome, deletion 22q11.2 syndrome, Fanconi anemia, Feingold syndrome, Fryns syndrome, MURCS association, oculo-auriculo-vertebral syndrome, Opitz G/BBB syndrome, Pallister-Hall syndrome, Townes-Brocks syndrome, and VACTERL with hydrocephalus. Though there are hints regarding causation, the aetiology has been identified only in a small fraction of patients to date, likely due to factors such as a high degree of clinical and causal heterogeneity, the largely sporadic nature of the disorder, and the presence of many similar conditions. New genetic research methods offer promise that the causes of VACTERL association will be better defined in the relatively near future. Antenatal diagnosis can be challenging, as certain component features can be difficult to ascertain prior to birth. The management of patients with VACTERL/VATER association typically centers around surgical correction of the specific congenital anomalies (typically anal atresia, certain types of cardiac malformations, and/or tracheo-esophageal fistula) in the immediate postnatal period, followed by long-term medical management of sequelae of the congenital malformations. If optimal surgical correction is achievable, the prognosis can be relatively positive, though some patients will continue to be affected by their congenital malformations throughout life. Importantly, patients with VACTERL association do not tend to have neurocognitive impairment.
Topics: Abnormalities, Multiple; Anal Canal; Anus, Imperforate; Esophagus; Female; Heart Defects, Congenital; Humans; Infant, Newborn; Kidney; Limb Deformities, Congenital; Male; Radius; Spine; Trachea; Tracheoesophageal Fistula
PubMed: 21846383
DOI: 10.1186/1750-1172-6-56 -
European Respiratory Review : An... Dec 2020Tracheo-oesophageal fistula (TOF) is a pathological connection between the trachea and the oesophagus that is associated with various underlying conditions including... (Review)
Review
Tracheo-oesophageal fistula (TOF) is a pathological connection between the trachea and the oesophagus that is associated with various underlying conditions including malignancies, infections, inhalation injuries and traumatic damage. As the condition spans multiple organ systems with varying aetiologies and acuities, TOF poses unique diagnostic and management challenges to pulmonologists, gastroenterologists and thoracic surgeons alike. Although stents have been a cornerstone in the management of TOF, there exists a large gap in our understanding of their efficacy and precise methodology, making stenting procedure both art and science. TOFs relating to underlying oesophageal or tracheal malignancies require advanced understanding of the airway and digestive tract anatomy, dimensions of the fistula, stent characteristics and types, and the interplay between the oesophageal stent and the airway stent if dual stenting procedure is elected. In this review article, we review the most up-to-date data on risk factors, clinical manifestations, diagnostic approaches, management methods and prognosis. Consequently, this article serves to evaluate current therapeutic strategies and the future directions in the areas of 3D-printed stents, over-the-scope clipping systems, tissue matrices and atrial septal closure devices.
Topics: Adult; Humans; Prognosis; Stents; Trachea; Tracheoesophageal Fistula
PubMed: 33153989
DOI: 10.1183/16000617.0094-2020 -
Respiratory Care Aug 2010Tracheostomy tubes are placed for a variety of reasons, including failure to wean from mechanical ventilation, inability to protect the airway due to impaired mental... (Review)
Review
Tracheostomy tubes are placed for a variety of reasons, including failure to wean from mechanical ventilation, inability to protect the airway due to impaired mental status, inability to manage excessive secretions, and upper-airway obstruction. A tracheostomy tube is required in approximately 10% of patients receiving mechanical ventilation and allows the patient to move to a step-down unit or long-term care hospital. The presence of a tracheostomy tube in the trachea can cause complications, including tracheal stenosis, bleeding, infection, aspiration pneumonia, and fistula formation from the trachea to either the esophagus or the innominate artery. Final removal of the tracheostomy tube is an important step in the recovery from chronic critical illness and can usually be done once the indication for the tube placement has resolved.
Topics: Critical Illness; Device Removal; Humans; Positive-Pressure Respiration; Tracheostomy; Ventilator Weaning
PubMed: 20667155
DOI: No ID Found -
The European Respiratory Journal Feb 2018Tracheal reconstruction is one of the greatest challenges in thoracic surgery when direct end-to-end anastomosis is impossible or after this procedure has failed. The... (Review)
Review
Tracheal reconstruction is one of the greatest challenges in thoracic surgery when direct end-to-end anastomosis is impossible or after this procedure has failed. The main indications for tracheal reconstruction include malignant tumours (squamous cell carcinoma, adenoid cystic carcinoma), tracheoesophageal fistula, trauma, unsuccessful surgical results for benign diseases and congenital stenosis. Tracheal substitutes can be classified into five types: 1) synthetic prosthesis; 2) allografts; 3) tracheal transplantation; 4) tissue engineering; and 5) autologous tissue composite. The ideal tracheal substitute is still unclear, but some techniques have shown promising clinical results. This article reviews the advantages and limitations of each technique used over the past few decades in clinical practice. The main limitation seems to be the capacity for tracheal tissue regeneration. The physiopathology behind this has yet to be fully understood. Research on stem cells sparked much interest and was thought to be a revolutionary technique; however, the poor long-term results of this approach highlight that there is a long way to go in this research field. Currently, an autologous tissue composite, with or without a tracheal allograft, is the only long-term working solution for every aetiology, despite its technical complexity and setbacks.
Topics: Allografts; Aorta; Humans; Prostheses and Implants; Plastic Surgery Procedures; Stem Cells; Thoracic Surgical Procedures; Tissue Engineering; Trachea; Tracheal Stenosis
PubMed: 29444919
DOI: 10.1183/13993003.02211-2017 -
Journal of Thoracic Disease Mar 2016Tracheal resection and reconstruction (TRR) and laryngotracheal resection and reconstruction (LTRR) is commonly performed for post-intubation tracheal stenosis, tracheal... (Review)
Review
Tracheal resection and reconstruction (TRR) and laryngotracheal resection and reconstruction (LTRR) is commonly performed for post-intubation tracheal stenosis, tracheal tumor, idiopathic laryngotracheal stenosis (ILTS), and tracheoesophageal fistula (TEF). Ninety-five percent of patients have a good result from surgery. Complications occur in ~20% of patients, of which half are anastomotic complications. Complications include granulation tissue formation, restenosis of the trachea, anastomotic separation, TEF and tracheoinnominate fistula (TIF), wound infection, laryngeal edema, and glottic dysfunction. Risk factors for anastomotic complication include diabetes, reoperation, previous tracheal appliance, and long-segment tracheal resection. Bronchoscopy should be part of the diagnostic workup when a complication is suspected. Anastomotic separation-the most feared complication of tracheal surgery-may present subtly with stridor and wound infection, or with respiratory distress and extremis. Prompt management is required to prevent devastating consequences. The airway should be secured, bronchoscopy should be performed to address the degree of separation, and the anastomosis should be revised if needed, usually with the addition of t-tube or tracheostomy. Anastomotic complications that are managed aggressively typically yield good results. More than half of such patients will eventually have a satisfactory airway. However, an anastomotic complication is associated with a thirteen-fold increase in the risk of death following tracheal resection.
PubMed: 26981267
DOI: 10.3978/j.issn.2072-1439.2016.01.86 -
Therapeutic Advances in Respiratory... Apr 2017Tracheoesophageal fistulas (TEFs) often occur with esophageal or bronchial carcinoma. Currently, we rely on implantation of delicate devices, such as self-expanding and... (Review)
Review
Tracheoesophageal fistulas (TEFs) often occur with esophageal or bronchial carcinoma. Currently, we rely on implantation of delicate devices, such as self-expanding and silicone stents, in the esophagus or trachea to cover the fistula and expand the stenosis in order to relieve patient pain. However, because each case is different, our approach may not be effective for every patient. Consequently, new devices and technology have emerged to address these situations, such as degradable stents, Amplatzer devices, endobronchial one-way umbrella-shaped valves, and transplantation of mesenchymal stem cells. Although some studies have shown such alternatives can be reasonable solutions in special cases, further development of other new and effectual techniques is of utmost importance.
Topics: Bronchial Neoplasms; Equipment Design; Esophageal Neoplasms; Humans; Pain; Stents; Tracheoesophageal Fistula
PubMed: 28391759
DOI: 10.1177/1753465816687518 -
Indian Journal of Surgical Oncology Mar 2022Thyroid cancer invading the trachea can be asymptomatic, but when tumour invasion reaches the mucosal surface, it causes bloody sputum and dyspnoea. The treatment plan... (Review)
Review
Thyroid cancer invading the trachea can be asymptomatic, but when tumour invasion reaches the mucosal surface, it causes bloody sputum and dyspnoea. The treatment plan for thyroid cancer is determined based on the site, depth, and extent of the invasion. Different from tumours arising from the tracheal mucosa, in thyroid cancer, invasion begins outside the airway and progresses toward the lumen, making it difficult to accurately diagnose the extent of the invasion even with bronchoscopy. Therefore, surgeons must determine the range of resection during surgery. Invasion reaching the tracheal mucosa requires full-thickness resection and is performed using tracheal window resection combined with tracheocutaneous fistula or tracheal sleeve resection followed by end-to-end anastomosis. The airway is safely secured with window resection, but closing the tracheal stoma often requires multi-stage reconstruction. Sleeve resection is an oncologically appropriate surgical method that can be completed in one stage, although there is a risk of serious complications associated with anastomotic dehiscence. Since well-differentiated thyroid cancer progresses slowly, some degree of survival can be expected even with incomplete resection. However, when shaving is performed for tumours with deep invasion that reaches the tracheal mucosa, the residual tumour tissue continues to grow steadily and eventually leads to airway stenosis. Since reoperation for tracheal resection is difficult, radical full-thickness resection should be performed in the initial surgery. Although this surgical intervention is far more demanding for both patients and surgeons than shaving, the procedure eventually improves patient's prognosis and quality of life.
PubMed: 35462665
DOI: 10.1007/s13193-021-01466-7